5612576 DEVELOPMENT AND IMPLEMENTATION OF NEUROPSYCHOLOGICAL SCREENING AND EVALUATION SERVICES FOR ADULTS LIVING WITH SICKLE CELL DISEASE: FINDINGS FROM A TWO-YEAR PILOT PROGRAM
Authors: J. Cavazos, W. Santos-Modesitt, M. Treadwell
Journal: HemaSphere (2023)

Background: Individuals with sickle cell disease (SCD) may experience diffuse brain injury, impacting neurocognitive development. They may also experience overt strokes and silent cerebral infarcts. These infarcts lead to impaired cognitive functioning across various domains including difficulties with aspects of executive functioning, working memory, processing speed, general intellectual abilities, and difficulties with learning. Research suggests the long-term consequences of these deficits in childhood are likely to have an impact on individuals’ lives as adults. Unfortunately, within the United States, neuropsychological screenings and evaluations are not considered a standard of care for SCD. Ensuring individuals with SCD have these services is critical for optimal care and improved health outcomes. Aims: Neurocognitive services are important for early identification, diagnosis, and treatment; and provide information about current level of functioning, including strengths and challenges. Neurocognitive data can inform strategies to increase health knowledge, adherence to appointments/medications, improve quality of life and prevent/reduce mortality rates. In July 2020, we were awarded a two-year grant to develop and implement a neuropsychological screening and evaluation program for adults living with SCD via an outpatient specialty clinic. Methods: Screening measures were used to detect cognitive dysfunction and challenges with attention and executive functioning. Fifty-one patients (18-73 years) were offered a screening, with 39 completing it. Fifteen patients were referred for further evaluation, with 13 completing a three-hour abbreviated neurocognitive evaluation to date. In this presentation, we will report results of these evaluations. Results: Ten patients were female, one transgender woman, and three were male. Ages ranged from 19-49 years (M = 28.5 years +/- 9.8); 12 were Black/African, and two biracial. Diagnoses were SCD-SS (n = 9), SCD-SC (n = 4), and one SCD-SB+ thalassemia. Five patients had had one or more strokes. Almost half (n = 7) performed in the lowest 10% of the normative population on a test of spatial visualization and motor skill. All performances on a test of visual-construction ability and visual memory were in the lowest 20% of the normative population, with six in the lowest one percent. Five performed in the average range or above on a test of cognitive slowing – the remainder scored very low on this test. In terms of areas of strength, many patients scored in the average range in areas such as verbal comprehension and perceptual reasoning, demonstrated persistence and had found ways to compensate for cognitive related difficulties. Summary: With all aspects of the neurocognitive evaluation taken together, we found that consistent with the literature, our patients evidenced challenges with processing speed, working memory, and aspects of executive functioning. While research on the impact of SCD on visual spatial/visual motor, and visual construction abilities is scant, our patients demonstrated notable deficits in this area, warranting further research. Conclusion: Neurocognitive services need to become a standard of care for SCD care. Additionally, as providers we need to better educate our patients on brain health throughout their lifespan and advocate for access to these types of services. References 1. Longoria et al, Br J Haematol 2022; 197(3): 260 2. Martin et al, Neuropsychological Rehabilitation 2020; 30(9): 1666 3. Noll et al, J Pediatr Psychol 2001; 26(2): 69 4. Schroeder et al, Am Fam Physician 2019; 99(2): 101 5. Vichinsky et al, JAMA 2010; 303(18): 1823